Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? A case series.

AIMS: To review the effect of total splenectomy on lung function and nutrition in children with cystic fibrosis related liver disease (CFLD) and associated portal hypertension. The stated indications for surgery and the short and long term risks of the procedure were also documented. METHOD: Over a 25 year period from January 1980 to June 2005, approximately 650 patients with cystic fibrosis (CF) were treated at the Royal Children's Hospital, Melbourne, Australia. Nine patients with CFLD who underwent a splenectomy during that time were identified and their medical records were reviewed. RESULTS: FEV1% predicted dropped by -16+/-11% in the two years pre-splenectomy. This contrasts with the increase in FEV1% predicted of 2+/-16% in the two years post-splenectomy (p = 0.05). The cumulative gain in WAZ score (DeltaWAZ pre) over the two years prior to splenectomy of 0.045+/-0.69 was not significantly different from the cumulative gain in WAZ score (DeltaWAZ post) for the two years after splenectomy of 0.15+/-0.36 (p = 0.65). The average age at splenectomy was 14.8 years (SD = 3 years). The average weight of an excised spleen was 983 g (SD = 414 g). There were no deaths associated with splenectomy. The median length of follow up post-splenectomy was 6.0 years (range 0.7-15.8). There were no episodes of bacterial peritonitis or overwhelming sepsis. CONCLUSIONS: Splenectomy may have a beneficial effect on lung function although this may not manifest itself until the second year post-splenectomy. Splenectomy in patients with CFLD appears to be a safe procedure.